Polycystic kidney disease pathology slide

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Polycystic Kidney Disease - Pathology

Gross Pathology

Fig. 46.8 - Greatly enlarged polycystic kidneys from ADPKD compared with a normal kidney (middle):

Fig. 12.23 (Robbins) - ADPKD (A,B) and ARPKD (C) and associated liver cysts (D):

ADPKD external surface (A), bisected (B), ARPKD (C), liver cysts (D)

Gross features of ADPKD:

Both kidneys reach enormous size - weights up to 4 kg each
On cut section: innumerable spherical cysts up to 3-4 cm in diameter distributed evenly between cortex and medulla
Cysts contain fluid that is clear, turbid, or hemorrhagic (dark brown/green = old blood)
Normal intervening parenchyma is essentially absent in end-stage disease
Fibrous bands encapsulate cysts on cut surface; fibrous tissue plasters the capsule

Gross features of ARPKD:

Smaller cysts giving a sponge-like appearance
Dilated, elongated channels oriented at right angles to the cortical surface, replacing cortex and medulla uniformly
Invariably bilateral

Histology / Microscopic Pathology

Fig. 46.9 - H&E histology of ADPKD cysts (x200):

Cyst histology in ADPKD: (A) papillary hyperplasia of cyst epithelium, (B) papillary microscopic adenoma

Key histologic features of ADPKD cysts:

Cysts arise from any nephron segment (tubules and collecting ducts); most studies indicate predominantly distal nephron and collecting duct origin
Cyst lining is variable and often atrophic (flattened cuboidal epithelium)
Hyperplastic changes are characteristic:
- (A) Papillary hyperplasia of cyst epithelium - frond-like projections into the cyst lumen
- (B) Papillary microscopic adenomas form within the cyst wall
Occasionally, Bowman's capsule gives rise to cysts - in these, glomerular tufts can be seen within the cystic space
Even in early disease with normal GFR: interstitial fibrosis, pre-glomerular vascular sclerosis (afferent arterioles + interlobular arteries), and macrophage/lymphocyte interstitial infiltrate
End-stage: tubulointerstitial fibrosis and arteriolar sclerosis are cardinal features; scant normal parenchyma in isolated patches

Fig. 45.6 (Brenner & Rector) - Scanning electron micrographs of ADPKD cyst epithelium:

SEM of cyst epithelium in ADPKD: panels A-F showing glomerular, proximal tubule, loop of Henle, distal tubule, and collecting duct origin epithelia

The 6 SEM panels show the diverse origin of cysts - epithelia morphologically resembling: (A) glomerular visceral layer, (B) proximal tubule brush border, (C) thick ascending limb, (D) distal tubule, (E) collecting duct principal cells, (F) medullary collecting duct. This variability confirms cysts arise from all nephron segments.

ARPKD histology:

Uniform lining of cuboidal cells (reflects collecting tubule origin throughout)
Uniformly small cysts; elongated channels perpendicular to cortical surface
Associated liver findings: multiple epithelium-lined hepatic cysts + portal bile duct proliferation (congenital hepatic fibrosis)

Pathogenesis Summary

Feature	ADPKD	ARPKD
Gene	PKD1 (chr 16p) ~85%; PKD2 (chr 4q) ~15%	PKHD1
Protein	Polycystin-1 / Polycystin-2	Fibrocystin
Location	Primary cilia (TRP channel complex)	Primary cilia
Mechanism	"Two-hit": somatic loss of second allele → loss of mechanosensing → ↑ proliferation, ↑ fluid secretion, ↓ calcium signaling, ↑ cAMP	Loss of fibrocystin → collecting duct dilatation
Prevalence	1:400-1000	1:20,000
Age at presentation	4th-5th decade	Perinatal/neonatal

The underlying defect is a ciliopathy - the polycystins (PC1 and PC2) reside in the primary cilia of tubular epithelial cells and sense fluid flow. Loss of PC1 function below a critical threshold disrupts calcium influx and planar polarity, triggering cyst expansion through increased mTOR/cAMP signaling.

Sources: Robbins & Kumar Basic Pathology, p. 525-526 | Comprehensive Clinical Nephrology, 7th Ed., p. 653-660 | Brenner and Rector's The Kidney, p. 1985-1988

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